Mario Naranjo, Erika B. Rosenzweig, Anna B. Hemnes, Miriam Jacob, Ankit Desai, Nicholas S. Hill, A. Brett Larive, J. Emanuel Finet, Jane Leopold, Evelyn Horn, Robert Frantz, Frans Rischard, Serpil Erzurum, Gerald Beck, Stephen C. Mathai, Paul M. Hassoun
Johns Hopkins University. Columbia University. Vanderbilt University Medical Center. Cleveland Clinic. University of Arizona College of Medicine. Tufts Medical Center. Brigham and Women’s Hospital and Harvard University. Weill Cornell University. Mayo Clinic.
United States
Pulmonary Circulation
Pulm Circ 2023; 13
DOI: 10.1002/pul2.12281
Abstract
The prevalence of acute vasodilator response (AVR) to inhaled nitric oxide (iNO) during right heart catheterization (RHC) is 12% in idiopathic pulmonary arterial hypertension (IPAH). AVR, however, is reportedly lower in other disease-associated pulmonary arterial hypertension (PAH), such as connective tissue disease (CTD). The prevalence of AVR in patients on PAH therapy (prevalent cases) is unknown. We sought to determine AVR prevalence in Group 1 PH in the PVDOMICS cohort of incident and prevalent patients undergoing RHC. AVR was measured in response to 100% O2 and O2 plus iNO, with positivity defined as (1) decrease in mean pulmonary artery pressure (mPAP) by ≥10 mmHg to a value ≤40 mmHg, with no change or an increase in cardiac output (definition 1); or (2) decrease in mPAP by ≥12% and pulmonary vascular resistance by ≥30% (definition 2). AVR rates and cumulative survival were compared between incident and prevalent patients. In 338 mainly prevalent (86%) patients, positive AVR to O2-only was <2%, and 5.1% to 16.9%, based on definition 1 and 2 criteria, respectively; following O2 + iNO. IPAH AVR prevalence (4.1%-18.7%) was similar to prior reports. AVR positivity was 7.7% to 15.4% in mostly CTD-PAH prevalent cases, and 2.6% to 11.8% in other PAH groups. Survival was 89% in AVR responders versus 77% in nonresponders from PAH diagnosis, and 91% versus 86% from PVDOMICS enrollment (log-rank test p = 0.04 and p = 0.05, respectively). In conclusion, AVR in IPAH patients is similar to prior studies. AVR in non-IPAH patients was higher than previously reported. The relationship between PAH therapy, AVR response, and survival warrants further investigation.
Category
Diagnostic Testing for Pulmonary Vascular Disease. Invasive Testing
Class I. Pulmonary hypertension Associated with a Favorable Response to Nitric Oxide and Calcium Channel Blockers
Age Focus: Adult Pulmonary Vascular Disease
Fresh or Filed Publication: Filed (PHiled). Greater than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes